Deterioration Of Interstitial Lung Disease Research Articles

Soluble CD206 levels correlate with disease deterioration and predict prognosis of anti-MDA5 antibody-positive dermatomyositis related interstitial lung disease.

The prognosis of anti-MDA5 antibody-positive dermatomyositis/clinically amyopathic dermatomyositis-associated interstitial lung disease (MDA5-DM/CADM-ILD) is poor. This study was to evaluate the effect of serum soluble CD206 (sCD206), a biomarker of macrophage activation, on predicting the interstitial lung disease (ILD) deterioration and prognosis for MDA5-DM/CADM-ILD. Forty-one patients diagnosed with MDA5-DM/CADM-ILD were retrospectively included. The clinical data were analyzed. Serum sCD206 levels were measured in 41 patients and 30 healthy controls. The relation between sCD206 levels and ILD deterioration was assessed. Receiver operating characteristic (ROC) curve was generated to determine the optimal cut-off value of sCD206 for predicting outcome. The association between sCD206 and survival was examined. The median serum sCD206 level in patients was significantly higher than healthy controls (464.1 ng/mL vs. 349.1 ng/mL, P= 0.002). In DM/CADM patients, the sCD206 level was significantly higher in patients with acute/subacute interstitial lung disease (AILD/SILD) than those with chronic interstitial lung disease (CILD) (539.2ng/mL vs. 309.4ng/mL, P= 0.005). The AUC of sCD206 was 0.885 for predicting mortality (95% CI 0.779-0.990). Patients were divided into two groups: sCD206 high level group (≥400 ng/mL) and sCD206 low level group (<400 ng/mL). Patients with sCD206 high level had significantly decreased survival rate than those with low level (25% vs. 88%, P< 0.001). The adjusted hazard ratio of sCD206 for mortality was 1.003 (adjusted for age and gender, P< 0.001), with sCD206 high level associated with higher death risk (HR 4.857, P= 0.006). Serum sCD206 might be a potential predictor of ILD deterioration and prognosis for Chinese patients with MDA5-DM/CADM-ILD.

Radiological evaluation of interstitial lung disease in patients with rheumatoid arthritis treated with abatacept or JAK inhibitors for 1 year

BackgroundWe evaluated the radiological deterioration of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) treated with abatacept (ABT) or Janus-kinase inhibitors (JAKi) for 1 year. MethodsThis study enrolled 84 patients with RA who had started treatment with ABT (48 patients) or JAKi (36 patients) between 2017 and 2020. All patients underwent chest CT before administration and at 1 year post-treatment. ResultsIn all patients, the rate of deterioration of ILD in ABT and JAKi was 4.2% and 5.6%, respectively (p = 0.847). In the patients with pre-existing ILD, the rates of deterioration of ILD in the ABT and JAKi groups were 10.5% and 18.2%, respectively (p = 0.611). Meanwhile, newly developed ILD did not occur in the two groups without pre-existing ILD. ConclusionThe present study demonstrated that the safety of pulmonary toxicity with ABT and JAKi treatments was similar in patients with RA. JAKi may be a potentially acceptable treatment option for patients with RA.

Clinical features, prognostic factors, and survival of patients with antisynthetase syndrome and interstitial lung disease.

ObjectiveThis study aimed to analyze the clinical features and prognostic factors of imaging progression and survival in patients with antisynthetase syndrome (ASS) complicated by interstitial lung disease (ILD) in a large Chinese cohort.MethodsMedical records, imaging, and serological data of 111 patients with ASS-ILD (positive for at least one of the following autoantibodies: anti-Jo1, anti-PL7, anti-PL12, and anti-EJ) from the Affiliated Yantai Yuhuangding Hospital of Qingdao University database were retrospectively investigated. According to the changes in high-resolution computed tomography (HRCT) outcomes at 1 year follow-up, Patients were categorized into three groups: the regression, stability, and deterioration groups. Univariate analysis was performed to evaluate the possible prognostic factors of ILD outcome and death, and multivariate analysis was performed to determine the independent predictors of ASS-ILD outcome and death by logistic regression.ResultsThe number of CD3-CD19+ cells and initial glucocorticoid dosage were correlated with imaging progression, and may be independent risk factors for ILD deterioration. Dyspnea as the first symptom, hypohemoglobinemia, the serum ferritin level, oxygen partial pressure at diagnosis, and different treatment types were important factors affecting survival, and the initial serum ferritin level may be an independent risk factor for survival.ConclusionsThe clinical characteristics of patients with ASS-ILD with different antisynthetase antibody subtypes are different. An increase in the CD3-CD19+ cell level is an independent risk factor for the deterioration of HRCT imaging. Early intensive treatment with high-dose glucocorticoids can effectively improve imaging prognosis of ILD. Patients with significantly elevated serum ferritin levels should be treated intensively.

COVID-19 causing acute deterioration of interstitial lung disease: a case report

BackgroundInterstitial lung disease (ILD) comprises a heterogeneous group of disorders characterized by multifocal diffuse lung involvement. Similarly, COVID-19 has varied multispectral organ involvement. Patients with underlying ILD and coexistent COVID-19 infection may lead to an acute blow to the already deceased lung.Case presentationA 58-year-old man presented with fever and cough with expectoration for the past 4 days associated with breathlessness. He was a smoker and alcoholic for the past 20 years. His saturation was low in room air around 84% and improved to 98% with 10 l/min of nasal oxygen. Further investigation shows acute deterioration of ILD.ConclusionCOVID-19 infection has a spectrum of manifestations. Acute deterioration of ILD is rarely reported in the literature. Etiology should be investigated further.

SAT0101 ABATACEPT IN RHEUMATOID ARTHRITIS ASSOCIATED-INTERSTITIAL LUNG DISEASE: SHORT TERM OUTCOME AND PROGNOSTIC FACTORS

Background:The prevalence of interstitial lung disease (ILD) in rheumatoid arthritis (RA) varies in the medical literature from 1% to 67% and is a major cause of mortality. Previous works have identified increased age, smoking and anticytrullinated protein antibodies (ACPA) titre as risk factors for RA-ILD (RA-ILD). Conventional treatments for RA may lead to a new onset or worsening of RA-ILD, so treatment should be identified to prevent the onset or exacerbation of RA-ILD. Studies have shown that abatacept (ABA) may improve the outcome of RA-ILD.Objectives:The aim of our study is to evaluate ABA effectiveness and safety in patients with RA-ILD.Methods:We enrolled RA-ILD patients who started treatment with ABA. All patients underwent thoracic high-resolution computed tomography (HRCT) at the beginning of ABA treatment and after 18 months of therapy. HRCT abnormalities were evaluated using a a computer-aided method (CaM). At each visit clinical, laboratory and respiratory function characteristics were collected and the Clinical Disease Activity Index (CDAI) and the Health Assessment Questionnaire Disability Index (HAQ-DI) for disease activity and functional disability were measured. The cohort was divided into three groups based on the CaM-HRCT results: patients with a lung fibrosis progression of 15% or more were defined as “worsened”, those with a reduction of 15% or more were defined as “improved”, all other patients were defined as “stable”. The 15% threshold of change, derived from the CaM assessment, results from the determination of the standard deviation of the mean value change after 18 months of follow-up of the cohort. The multivariate regression model was used to assess the strength of the association between RA characteristics and HRCT response to ABA.Results:Forty-four patients (81% women) with a mean age of 59.1±8.0 years and a mean duration of the disease of 7.5±3.1 years were recruited. Twenty-three (52.3%) patients were positive for ACPA and 28 (63.6%) for rheumatoid factor. Five patients (11.4%) showed deterioration of ILD, 31 (70.6%) were in the “stable” group, and 7 patients (16.0%) experienced improvement over a mean follow-up period of 18 months. The factors related to ILD deterioration were use of methotrexate (MTX) (p=0.0078), and current smoking habit (p=0.0054), according to multivariate regression analysis (Table).Conclusion:Treatment with ABA is associated with an RA-ILD stability or improvement rate of 86.6% of patients, while the worsening rate is 11.4%. Concomitant treatment with MTX and current smoking habit are factors associated with RA-ILD worsening. MTX discontinuation and smoking cessation should be strongly promoted in patients with RA-ILD.

Long-term deterioration of interstitial lung disease in patients with rheumatoid arthritis treated with abatacept

Objective: To examine the deterioration of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA) treated with abatacept over the long-term.Methods: We examined 131 patients with RA who had been treated with abatacept for more than 1 year. All patients underwent high-resolution computed tomographic (HRCT) scanning of the chest before administration of abatacept, and we examined deterioration of ILD over a follow-up period after administration of abatacept was initiated.Results: Eleven patients (8.4%) showed deterioration of ILD over a mean follow-up period of 47.8 months. The factors related to ILD deterioration were use of methotrexate (MTX) [odds ratio 12.75, 95% confidence interval (CI) 1.09–148.77], and change in Krebs von-den Lungen-6 (odds ratio 1.00, 95% CI 1.00–1.01), according to multivariate logistic regression analysis.Conclusion: MTX in patients with RA treated with abatacept was a risk factor for deterioration of ILD. Discontinuation of MTX should be considered one of treatment reduction to prevent the deterioration of ILD.

Predictive factors for long-term outcome in polymyositis/dermatomyositis-associated interstitial lung diseases.

Interstitial lung disease (ILD) is strongly associated with polymyositis (PM), dermatomyositis (DM), and clinically amyopathic dermatomyositis (CADM). It is also related to mortality. Previous studies have highlighted that the acute form of PM/DM/CADM-associated ILD (PM/DM/CADM-ILD) has a poor short-term prognosis. However, little is known about the long-term clinical features of patients with PM/DM/CADM-ILD. The aim of the present study is to clarify the clinical characteristics and the predictive factors for long-term outcomes in patients with PM/DM/CADM-ILD. Thirty-four patients with PM/DM/CADM-ILD who were followed up for more than 12 months were analyzed retrospectively. The patients were classified as "stable" or "deterioration" according to respiratory symptoms, serial changes in forced vital capacity (FVC) or arterial oxygen pressure, and radiologic findings during the follow-up period. Twenty-six patients (76%) were in the stable group and eight patients (24%) were in the deterioration group. Home oxygen therapy was performed in six cases in the deterioration group because of chronic respiratory failure due to progression of ILD. The deterioration group, in comparison to the stable group, had a significantly lower %FVC and a higher positive rate for the anti-PL-7 antibody. Multivariate logistic regression analysis revealed that a positive anti-PL-7 antibody test and a lower %FVC were independently associated with deterioration during long-term follow-up. Patients with PM/DM/CADM-ILD are at risk for chronic respiratory failure due to the deterioration of ILD during long-term follow-up. The presence of anti-PL-7 antibody and a lower %FVC at initial diagnosis may predict long-term deterioration in patients with PM/DM/CADM-ILD.

Serum KL-6 and surfactant protein-D as monitoring and predictive markers of interstitial lung disease in patients with systemic sclerosis and mixed connective tissue disease.

Interstitial lung disease (ILD) is frequent complication of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD). The disease is heterogeneous, and its outcome is unpredictable. Some patients have severe and progressive deterioration of ILD, which is the leading cause of mortality. We aimed to determine whether serum levels of Krebs von den Lungen-6 (KL-6) and surfactant protein-D (SP-D) correlate with SSc/MCTD-associated ILD activity. We retrospectively analyzed the medical records of 40 patients with SSc/MCTD-associated ILD: 29 patients with SSc and 11 patients with MCTD. Measurement of serum KL-6 and SP-D levels, pulmonary function tests, and high-resolution computed tomography (HRCT) performed in parallel were reviewed. Serum KL-6 correlated positively with diffusing capacity of the lung for carbon monoxide (DLCO) (% predicted) and disease extent on HRCT, and the changes in serum levels of KL-6 were significantly related to the changes in forced vital capacity (FVC) in SSc/MCTD-associated ILD. On the other hand, multivariate logistic regression analyses with calculation of the area under the curve of the receiver-operating characteristic curve suggested that a higher serum level of SP-D was a significant predictor of FVC decline in SSc/MCTD-associated ILD. Our study suggests that serum KL-6 can be a useful monitoring tool of SSc/MCTD-associated ILD activity. In contrast, serum SP-D may be a significant predictor of potential FVC decline in the short term.

Interstitial lung disease in primary Sjögren's syndrome.

Interstitial lung disease (ILD) has been reported in 3 to 11% of patients with primary Sjögren's syndrome (pSS). The aims of this retrospective multicenter study were to: 1) analyze characteristics and outcome of ILD in pSS; and 2) evaluate predictive factors associated with ILD onset and deterioration. Twenty-one of 263 patients with pSS (8%) developed ILD. ILD onset preceded pSS diagnosis (n=5), was concurrently identified in association with pSS (n=6) and developed after pSS onset (n=9). Presenting ILD manifestations were: acute/subacute (n=11) onset of ILD, symptomatic progressive onset of ILD (n=5), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT-scan (n=5). ILD therapy included: steroids (n=21), cyclophosphamide (n=1), azathioprine (n=4) and rituximab (n=1). The course of ILD was as follows: improvement (15.8%), stabilization (47.4%) or deterioration (36.8%). Predictive parameters of ILD onset were: older age (p=0.044), Raynaud's phenomenon (p=0.001) and esophageal involvement (p=0.001). Factors associated with ILD deterioration were: older age (p=0.038) and esophageal involvement (p=0.038). Thus, this study underscores the poor outcome of ILD during pSS; thus, systematic screening of pulmonary involvement is required in pSS patients, resulting in both diagnosis and management at early stage of ILD. We also suggest that patients presenting predictive factors of ILD deterioration may need a closer follow-up and a more aggressive therapy.

Interstitial Lung Disease in Anti–Jo‐1 Patients With Antisynthetase Syndrome

To assess the outcome of interstitial lung disease (ILD) in anti-Jo-1 patients with antisynthetase syndrome, determine predictive variables of ILD deterioration in these patients, and compare features of anti-Jo-1 patients with and without ILD. Ninety-one anti-Jo-1 patients were identified by medical records search in 4 medical centers. All of these patients had undergone pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) scans. Sixty-six patients (72.5%) had ILD. Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n = 12), progressive onset of lung signs (n = 35), and asymptomatic patients exhibiting abnormalities consistent with ILD on PFTs and HRCT scans (n = 19). Sixteen patients had resolution of ILD; 39 and 11 patients experienced improvement and deterioration of ILD, respectively. ILD led to decreased functional status, since 29.8% of patients exhibited a marked reduction of activities due to ILD and 13.6% had respiratory insufficiency requiring oxygen therapy; 5 of 6 patients died due to ILD complications. Predictive parameters of ILD deterioration were HRCT scan pattern of usual interstitial pneumonia, respiratory muscle involvement, and age ≥55 years. Furthermore, anti-Jo-1 patients with ILD, compared with those without, more frequently exhibited mechanic's hands and lower creatine kinase levels. Our findings confirm that ILD is a frequent complication in anti-Jo-1 patients, resulting in high morbidity. We suggest that patients with predictive factors of ILD deterioration may require more aggressive therapy. Finally, anti-Jo-1 patients with ILD, compared with those without, may exhibit a particular clinical phenotype.

Clinical manifestations and outcome of anti-PL7 positive patients with antisynthetase syndrome

BackgroundThe aims of the present study were to determine both clinical manifestations and outcome of anti-PL7 patients with antisynthetase syndrome (ASS). MethodsThe medical records of 15 consecutive anti-PL7 patients with biopsy proven ASS were retrospectively analyzed without prior selection. ResultsAnti-PL7 patients exhibited polymyositis (n=14) and dermatomyositis (n=1); extra-pulmonary manifestations of ASS included: Raynaud's phenomenon (40%), mechanic's hands (33.3%), joint impairment (26.7%), pericardial effusion (20%) and esophageal/gastrointestinal involvement (20%). The outcome of myositis was as follows: remission/improvement (91.7%) and deterioration (8.3%). Fourteen patients (93.3%) experienced interstitial lung disease (ILD). ILD preceded ASS diagnosis (n=5), was identified concomitantly with ASS (n=8) and occurred after ASS diagnosis (n=1). Patients could be divided into 3 groups according to their presenting lung manifestations: acute onset of lung disease (n=1), progressive onset of lung signs (n=11) and asymptomatic patients exhibiting abnormalities consistent with ILD on PFT and HRCT-scan (n=2). No patient had resolution of ILD, whereas 64.3% and 35.7% experienced improvement and deterioration of ILD, respectively. ILD resulted in respiratory insufficiency requiring O2 therapy in 14.3% of cases. Two patients died. Predictive parameters of ILD deterioration were: DLCO<45% at ILD diagnosis and HRCT-scan pattern of usual interstitial pneumonia (UIP). ConclusionOur series mainly underscores that ILD is frequent in anti-PL7 patients, leading to high morbidity. Our study further suggests that patients with predictive factors of ILD deterioration may require more aggressive therapy, especially the group of patients with DLCO<45% at ILD diagnosis and UIP pattern on HRCT-scan.

O1-033 - Acute Exacerbation of Pre-Existing Interstitial Lung Disease (ILD) in Patients with Lung Cancer under Various Treatments

ABSTRACT Background Acute deterioration of ILD for unknown causes, sometimes called as acute exacerbation (AE), can occur at any point in the course of ILD. However, little is known about its incidence and prognostic significance in lung cancer pts with pre-existing ILD, who receive various treatments; chemotherapy, surgery, palliative radiotherapy, and best supportive care (BSC). Methods A total of 242 subjects (6.9% of all) were retrospectively identified to have pre-existing ILD by computed tomography (CT) from a sum of 3524 pts who had been hospitalized for lung cancer treatment at 8 institutions during 2004 to 2009. CT images of all the eligible pts were centrally reviewed. Univariate and multivariate analyses were performed using a Cox proportional hazard model to examine the potential role of any prognostic factors for overall survival (OS) from the initial lung cancer diagnosis. Results Pts' characteristics were: male/female = 217/25; median age (range) = 73 (42-98) yrs.; smoking status: ever/never = 223/19; Performance Status: 0/1/2/3/4 = 74/121/23/19/5; Stage I/II/III/IV = 48/10/98/86; Histology: adeno/squamous/large/NOS/small = 90/75/6/19/52; CT pattern: usual interstitial pneumonia (UIP)/non-UIP = 118/124; extent of normal lung on baseline CT: 10-50%/60-90% = 154/88; pre-existing emphysema: yes/no = 178/64. AE occurred in 71 of 242 pts (29%) overall; 56 of 147 pts (38%) with chemotherapy, 6 of 38 pts (16%) with surgery, 2 of 17 pts (12%) with palliative radiotherapy, and 5 of 36 pts (14%) with BSC alone, and chemotherapy was an independent risk factor for the occurrence of AE (P Conclusions The occurrence of AE is not rare in the lung cancer treatment, particularly during chemotherapy, and it is a factor for poor prognosis in pts with pre-existing ILD. Disclosure All authors have declared no conflicts of interest.

1538P - Acute Exacerbation of Pre-Existing Interstitial Lung Disease (ILD) in Patients (PTS) with Lung Cancer Under Various Treatments

ABSTRACT Background Acute deterioration of ILD for unknown causes, sometimes called as acute exacerbation (AE), can occur at any point in the course of ILD. However, little is known about its incidence and prognostic significance in lung cancer pts with pre-existing ILD, who receive various treatments; chemotherapy, surgery, palliative radiotherapy, and best supportive care (BSC). Methods A total of 242 subjects (6.9% of all) were retrospectively identified to have pre-existing ILD by computed tomography (CT) from a sum of 3524 pts who had been hospitalized for lung cancer treatment at 8 institutions during 2004 to 2009. CT images of all the eligible pts were centrally reviewed. Univariate and multivariate analyses were performed using a Cox proportional hazard model to examine the potential role of any prognostic factors for overall survival (OS) from the initial lung cancer diagnosis. Results Pts' characteristics were: male/female = 217/25; median age (range) = 73 (42-98) yrs.; smoking status: ever/never = 223/19; Performance Status: 0/1/2/3/4 = 74/121/23/19/5; Stage I/II/III/IV = 48/10/98/86; Histology: adeno/squamous/large/NOS/small = 90/75/6/19/52; CT pattern: usual interstitial pneumonia (UIP)/non-UIP = 118/124; extent of normal lung on baseline CT: 10-50%/60-90% = 154/88; pre-existing emphysema: yes/no = 178/64. AE occurred in 71 of 242 pts (29%) overall; 56 of 147 pts (38%) with chemotherapy, 6 of 38 pts (16%) with surgery, 2 of 17 pts (12%) with palliative radiotherapy, and 5 of 36 pts (14%) with BSC alone, and chemotherapy was an independent risk factor for the occurrence of AE (P Conclusions The occurrence of AE is not rare in the lung cancer treatment, particularly during chemotherapy, and it is a factor for poor prognosis in pts with pre-existing ILD. Disclosure All authors have declared no conflicts of interest.

Short‐term and long‐term outcomes of interstitial lung disease in polymyositis and dermatomyositis: A series of 107 patients

This study was undertaken to assess the characteristics and outcome of interstitial lung disease (ILD) in polymyositis/dermatomyositis (PM/DM) and to determine variables predictive of ILD deterioration in PM/DM. Among 348 consecutive patients with PM/DM, 107 patients with ILD were identified by medical records search in 4 medical centers. All patients underwent pulmonary function tests (PFTs) and pulmonary high-resolution computed tomography (HRCT) scan. ILD onset preceded PM/DM clinical manifestations in 20 patients, was identified concurrently with PM/DM in 69 patients, and occurred after PM/DM onset in 18 patients. Patients with ILD could be divided into 3 groups according to their presenting lung manifestations: patients with acute lung disease (n = 20), patients with progressive-course lung signs (n = 55), and asymptomatic patients with abnormalities consistent with ILD evident on PFTs and HRCT scan (n = 32). We observed that 32.7% of the patients had resolution of pulmonary disorders, whereas 15.9% experienced ILD deterioration. Factors that predicted a poor ILD prognosis were older age, symptomatic ILD, lower values of vital capacity and diffusing capacity for carbon monoxide, a pattern of usual interstitial pneumonia on HRCT scan and lung biopsy, and steroid-refractory ILD. The mortality rate was higher in patients with ILD deterioration than in those without ILD deterioration (47.1% versus 3.3%). Our findings indicate that ILD results in high morbidity in PM/DM. Our findings also suggest that more aggressive therapy may be required in PM/DM patients presenting with factors predictive of poor ILD outcome.

Recent results of postoperative mortality for surgical resections in lung cancer

Background Changes in the postoperative mortality rates and causes of death for lung cancer surgery at the specialized hospital for cancer in Tokyo, Japan during the last 16 years were investigated. Methods Data on 3,270 consecutive patients who underwent pulmonary resection for primary lung cancer between January 1987 and December 2002 at the National Cancer Center Hospital were retrospectively analyzed. The postoperative 30-day and in-hospital mortality rates and causes of death after pulmonary resection for lung cancer were investigated. Patients were divided into two period groups of almost equal number, the early (1,615 patients from 1987 to 1996) and the late (1,655 patients from 1997 to 2002) periods. Results Fifty-eight operative and postoperative deaths occurred during the last 16 years. Thirty-day and in-hospital mortality were 0.6% (21/3,270) and 1.6% (58/3,270), respectively. During the last 6-year period, 30-day and in-hospital mortality were 0.5% (8/1,655) and 0.8% (21/1,655), respectively. The difference was significant between the 30-day/in-hospital mortality for pneumonectomy (3.1%/5.9%) and lobectomy (0.3%/1.3%) ( p < 0.0001/ p < 0.0001). The difference in mortality between lobectomy and segmentectomy or a lesser resection was not significant. The 58 deaths were caused by pneumonia/acute respiratory distress syndrome (ARDS) (36%, n = 21), bronchopleural fistula (BPF)/empyema (33%, n = 19), cerebrovascular accident (10%, n = 6), cardiac-related event (7%, n = 4), and others (14%, n = 8). The most frequent cause of death in the early period was BPF/empyema (18/45, 40%), while that in the late period was pneumonia/ARDS (6/13, 46%). Among the pneumonia/ARDS deaths in the late period (n = 6), 5 (83%) were due to acute deterioration of interstitial lung disease after lobectomy. Conclusions Recent postoperative mortality rates (30-day, 0.5%; in-hospital, 0.8%) in the treatment of lung cancer are quite acceptable. Special care must be taken for the patient after pneumonectomy, as reported by others. Furthermore, even after lobectomy, proper management of the patient with acute deterioration of interstitial lung disease will be required to improve the future outcome.

Interstitial lung disease in polymyositis and dermatomyositis.

To assess prevalence, characteristics, and long-term outcome of interstitial lung disease (ILD) in polymyositis (PM) and dermatomyositis (DM). To determine predictive variables of ILD course in PM/DM, and to define both clinical and biochemical features associated with ILD onset in PM/DM. The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. Thirty-six PM/DM patients (23.1%) developed ILD. We observed that 19.4% of patients with ILD had resolution of pulmonary disorders, whereas 25% experienced ILD deterioration. Morbidity and mortality rates were as high as 13.9% and 36.4%, respectively, in PM/DM patients with ILD. Parameters of PM/DM that related to ILD poor outcome were identified as follows: Hamman-Rich-like pattern, initial diffusing capacity of carbon monoxide <45%, neutrophil alveolitis, and histologic usual interstitial pneumonia. Additionally, for the group with ILD, polyarthritis, higher values of erythrocyte sedimentation rate and C-reactive protein, presence of anti-Jo-1 antibody, and characteristic microangiopathy were significantly more frequent. Our series underlines the high frequency of ILD in PM/DM patients, resulting in increased morbidity and mortality rates. It also indicates that PM/DM patients should routinely be screened for ILD, even those patients without anti-Jo-1 antibody, because 69% of our ILD patients were seronegative for the anti-Jo-1 antibody. Our findings further suggest that PM/DM patients presenting with factors predictive of ILD poor outcome may require more aggressive therapy.